Abstract：
To measure visual fields using two-color dark-adapted chromatic perimetry in a subset of participants in the Rate of Progression of USH2A-related Retinal Degeneration (RUSH2A), a study of USH2A-mediated syndromic (USH2) and autosomal recessive nonsyndromic retinitis pigmentosa, determine percentage retaining rod function, and explore relationships between dark-adapted visual fields (DAVF) and rod function from ERG and full-field stimulus thresholds (FST).
Full-field rod mean sensitivity, number of rod loci, maximum sensitivity, DAVF full-field hill of vision (DAVF VTOT), and 30° hill of vision (DAVF V30) were measured in one eye for DAVF ancillary study participants (n = 49). Loci where cyan relative to red sensitivity was more than 5 dB on dark-adapted chromatic perimetry were considered rod mediated. Correlation coefficients between the DAVF measures and standard clinical measures were estimated, as were kappa statistics (κ) for agreement between DAVF and other measures of rod function.
Of 49 participants tested with DAVF, 38 (78%) had evidence of rod function, whereas 15 (31%) had measurable rod ERGs. DAVF maximum sensitivity was highly correlated with FST white thresholds (r = -0.80; P < .001). Although not statistically significant, the number of rod loci and DAVF VTOT were lower in eyes with longer disease duration by 0.82 (95% confidence interval, -1.76, 0.12) loci/year and 0.59 (95% confidence interval, -1.82, 0.64) dB-steradians/year, respectively.
Rod-mediated function on FST and DAVF is present in many patients with symptomatic USH2A-related retinal degeneration, including some without measurable rod ERGs. RUSH2A longitudinal data will determine how these measures change with disease progression and whether they are useful for longitudinal studies in inherited retinal degenerations.
Full-field rod mean sensitivity, number of rod loci, maximum sensitivity, DAVF full-field hill of vision (DAVF VTOT), and 30° hill of vision (DAVF V30) were measured in one eye for DAVF ancillary study participants (n = 49). Loci where cyan relative to red sensitivity was more than 5 dB on dark-adapted chromatic perimetry were considered rod mediated. Correlation coefficients between the DAVF measures and standard clinical measures were estimated, as were kappa statistics (κ) for agreement between DAVF and other measures of rod function.
Of 49 participants tested with DAVF, 38 (78%) had evidence of rod function, whereas 15 (31%) had measurable rod ERGs. DAVF maximum sensitivity was highly correlated with FST white thresholds (r = -0.80; P < .001). Although not statistically significant, the number of rod loci and DAVF VTOT were lower in eyes with longer disease duration by 0.82 (95% confidence interval, -1.76, 0.12) loci/year and 0.59 (95% confidence interval, -1.82, 0.64) dB-steradians/year, respectively.
Rod-mediated function on FST and DAVF is present in many patients with symptomatic USH2A-related retinal degeneration, including some without measurable rod ERGs. RUSH2A longitudinal data will determine how these measures change with disease progression and whether they are useful for longitudinal studies in inherited retinal degenerations.
摘要：
在USH2A相关视网膜变性进展率（RUSH2A）的一部分参与者中使用双色深色适应彩色视野法测量视野，USH2A介导的综合征（USH2）和常染色体隐性遗传的非综合征性视网膜色素变性的研究，确定保持杆功能的百分比，并从ERG和全场刺激阈值（FST）探讨暗适应视野（DAVF）与视杆功能之间的关系。
全场棒平均灵敏度，棒基因座的数量，最大灵敏度,DAVF全视野山（DAVFVTOT），对DAVF辅助研究参与者(n=49)的一只眼睛测量了30°视丘(DAVFV30)。在暗适应的彩色视野检查中，青色相对于红色的灵敏度超过5dB的基因座被认为是杆状介导的。估算了DAVF措施与标准临床措施之间的相关系数，DAVF和其他杆函数度量之间的一致性也是kappa统计(κ)。
在DAVF测试的49名参与者中，38(78%)有杆功能的证据，而15(31%)有可测量的棒ERGs。DAVF最大灵敏度与FST白阈值高度相关(r=-0.80;P<.001)。虽然没有统计学意义，在病程较长的眼睛中，杆基因座和DAVFVTOT的数量减少了0.82（95％置信区间，-1.76，0.12)基因座/年和0.59(95%置信区间，-1.82,0.64)dB-steradians/年，分别。
在许多有症状的USH2A相关视网膜变性患者中存在FST和DAVF的Rod介导的功能，包括一些没有可测量的杆ERG。RUSH2A纵向数据将确定这些措施如何随疾病进展而变化，以及它们是否可用于遗传性视网膜变性的纵向研究。
全场棒平均灵敏度，棒基因座的数量，最大灵敏度,DAVF全视野山（DAVFVTOT），对DAVF辅助研究参与者(n=49)的一只眼睛测量了30°视丘(DAVFV30)。在暗适应的彩色视野检查中，青色相对于红色的灵敏度超过5dB的基因座被认为是杆状介导的。估算了DAVF措施与标准临床措施之间的相关系数，DAVF和其他杆函数度量之间的一致性也是kappa统计(κ)。
在DAVF测试的49名参与者中，38(78%)有杆功能的证据，而15(31%)有可测量的棒ERGs。DAVF最大灵敏度与FST白阈值高度相关(r=-0.80;P<.001)。虽然没有统计学意义，在病程较长的眼睛中，杆基因座和DAVFVTOT的数量减少了0.82（95％置信区间，-1.76，0.12)基因座/年和0.59(95%置信区间，-1.82,0.64)dB-steradians/年，分别。
在许多有症状的USH2A相关视网膜变性患者中存在FST和DAVF的Rod介导的功能，包括一些没有可测量的杆ERG。RUSH2A纵向数据将确定这些措施如何随疾病进展而变化，以及它们是否可用于遗传性视网膜变性的纵向研究。
